NSP OF INTERLOBULAR BILE DUCTS
 
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NSP OF INTERLOBULAR BILE DUCTS

 

DEFINITION:

A disorder characterized by a paucity of interlobular bile ducts resulting in hepatocellular cholestasis.

EPIDEMIOLOGY:

  • incidence: rare (about 50 cases reported)
  • age of onset:
    • neonatal period
  • risk factors:
    • sporadic

PATHOGENESIS:

1. Background

  • a disorder causing a direct hyperbilirubinemia and jaundice due to persistent intrahepatic cholestasis
  • there are 2 groups of disorders associated with a paucity of the intrahepatic bile ducts (IBD):
  • 1. Syndromatic Paucity of the IBD (Alagille Syndrome)
  • 2. Nonsyndromatic Paucity of the IBD
  • 1. Idiopathic
    • Nonsyndromatic Paucity (NSP) of Interlobular
    • Bile Ducts
  • 2. Secondary
  • 1. Infectious
  • TORCH infections
  • 2. Genetic
  • Trisomy 21, 18
  • 3. Metabolic
  • Alpha-1-Antitrypsin Deficiency
  • Cystic Fibrosis
  • Zellweger's Syndrome

2. Etiology

  • unknown; inflammation of the intrahepatic bile ducts may lead to paucity of these ducts

CLINICAL FEATURES:

1. Gastrointestinal Manifestations

1. Cholestasis

  • poor feeding
  • lethargy
  • hepatomegaly +/- splenomgaly
  • jaundice
  • irritability (pruritis)
  • steatorrhea
  • vomiting

2. Complications

  • malabsorption of fats and fat-soluble vitamins (D,E,A,K)
  • failure to thrive
  • xanthomatosis (as early as 18 months of age)
  • hepatic fibrosis with portal hypertension
  • cirrhosis with liver failure

INVESTIGATIONS:

1. Serum

  • conjugated hyperbilirubinemia
  • elevated bile salts, lipids, cholesterol, AST, ALT
  • vitamin D,E,A,K deficiencies

2. For Malabsorption

  • elevated 72 hour fecal fat content

3. Liver Biopsy

  • bile duct paucity, portal fibrosis, giant cell transformation, hepatocellular cholestasis

MANAGEMENT:

1. Medical

1. Symptomatic

  • cholestyramine to bind bile salts and reduce pruritis
  • supplementation with fat-soluble vitamins (D,E,A,K)
  • promote bile flow with phenobarbital or rifampin

2. Surgical

  • liver transplantation for cirrhosis

3. Prognosis

  • highly variable ranging from complete resolution of symptoms to intractable pruritis (even in the absence of jaundice) to cirrhosis in up to 55% of cases

 

 

 

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